Diagnosis and treatment of male pseudohermaphroditism (report of 21 cases) / 中华泌尿外科杂志

ABSTRACT

Objective To report and summarize the improvement of diagnosis and treatment of male pseudohermaphroditism. Methods Among the 21 cases (mean age of 13 years,range from 6 to 31 years),19 were socially female sex and 2 were male sex.Visual clinical examination indicated abnormal external genitalia,lumps on both sides of inguina or labia majora pudendi.Genetic examination showed that the sex chromosome karyotypes of all cases were 46,XY.Before operation diagnoses were established in 20 cases by blood biochemistry,B-ultrasound and CT examination with misdiagnosis in only 1 case. Results All the 21 cases received operation.Female features were retained in 19 cases and male features in 2.Of them 15 cases were followed up for 3 to 8 years.Of the 15 cases 14 were treated by hormone replacement (oral stilbestrol)with obvious improvement of secondary sexual character.Of the 14 cases 11 had normal prepuberal physical development.The appearances of the external genitalia were satisfactory.One patient who maintained male sex had obvious growth of breasts during puberty. Conclusions Early diagnosis and treatment are very important to the therapeutic effects and the life quality of the patients.It is suggested that operation of maintaining female sex can be preferred as treatment of choice for male pseudohermaphroditism.